Moyamoya disease presenting as intracerebral hemorrhage during pregnancy: case report and review of the literature.

Intracranial hemorrhage during pregnancy is rare. The authors present a case of moyamoya disease associated with pregnancy. A 32-year-old woman who was at 32 weeks of gestation experienced sudden severe headache followed by loss of consciousness. Computed tomography revealed an intracerebral hematoma in the left temporal lobe, and left carotid angiograms revealed moyamoya disease. The patient underwent emergency craniotomy, the hematoma was removed, and encephaloduroarteriosynangiosis was performed. The patient showed good recovery and, 2 months later, delivered a child vaginally without any trouble. Most intracranial hemorrhages during pregnancy result from cerebral aneurysms or arteriovenous malformations (AVMs), and few cases due to moyamoya disease have been reported. It is generally believed that subarachnoid hemorrhage due to cerebral aneurysms is likely to occur during the middle trimester of gestation, with the risk increasing progressively toward the third trimester. This may be explained by the fact that the cardiac output increases rather acutely from the first to the middle trimester. There seems to be no significant correlation between the stage of pregnancy and the occurrence of hemorrhage due to AVM. Most authors think that the operative indications for intracranial hemorrhage during pregnancy should be the same as for the nonpregnant state.